rspНаучно-практическая ревматологияRheumatology Science and Practice1995-44841995-4492IMA-PRESS, LLC10.14412/1995-4484-2016-463-468rsp-2277Research ArticleОБЗОРЫСиндром Когана – новая нозология в современной классификации системных васкулитов. Клиническое наблюдение и обзор литературыCogan's syndrome is a new nosological entity in the current classification of systemic vasculitides: A clinical case and a review of literatureБекетоваТ. В.BeketovaT. V.

115522 Москва, Каширское шоссе, 34А 

34A, Kashirskoe Shosse, Moscow 115522 

TVBEKTVBEK@rambler.ruФГБНУ Научно- исследовательский институт ревматологии им. В.А. Насоновой, МоскваРоссияV.A. Nasonova Research Institute of Rheumatology, MoscowRussian Federation201620102016544463468Copyright © Бекетова Т.В., 20162016Бекетова Т.В.Beketova T.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://rsp.mediar-press.net/rsp/article/view/2277

Представлено клиническое наблюдение типичного варианта синдрома Когана (СК) – новой нозологической формы современной классификации системных васкулитов (СВ), принадлежащей к группе вариабельных васкулитов. В обзоре литературы подробно освещены проблемы диагностики и лечения этого редкого заболевания.

СК характеризуется воспалительным поражением глаз (интерстициальный кератит, увеит, эписклерит) и патологией органа слуха (нейросенсорная тугоухость, вестибулярные нарушения) с возможным развитием васкулита другой локализации, аортита, поражения аортального и митрального клапанов. Системное поражение с вовлечением ЛОР-органов и глаз требует исключения гранулематоза с полиангиитом (Вегенера) и дифференциальной диагностики с широким спектром заболеваний. Представленные данные подчеркивают необходимость междисциплинарного сотрудничества ревматологов, оториноларингологов, аудиологов, офтальмологов, кардиологов для совершенствования диагностики и лечения этой формы СВ. 

The paper describes a clinical case of the typical variant of Cogan's syndrome (CS), a new nosological entity in the current classification of systemic vasculitides (SV), which belongs to a group of variable vasculitides. The literature review highlights in detail the problems of the diagnosis and treatment of this rare disease.

CS is characterized by inflammatory eye involvement (interstitial keratitis, uveitis, and episcleritis) and hearing problems (sensorineural hearing loss, vestibular disorders) with the possible development of vasculitis at other sites, aortitis, and aortic or mitral lesions. A systemic lesion involving the ears and eyes necessitates to rule out granulomatosis with polyangiitis (Wegener's) and to make a differential diagnosis with a wide range of diseases. The given data underline the need for the interdisciplinary collaboration of rheumatologists, otorhinolaryngologists, audiologists, oculists, and cardiologists in order to improve the diagnosis and treatment of this form of SV. 

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The authors declare that there are no conflicts of interest present.